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Annals of Hematology (Online First™)
Features of extramedullary myeloma relapse: high proliferation, minimal marrow involvement, adverse cytogenetics: a retrospective single-center study of 24 cases
Mon, 30 Jan 2012 06:32:07 -0000
Abstract Extramedullary (e) relapse in multiple myeloma (MM) has an adverse prognosis, but knowledge concerning biological features and preferred treatment is scarce. We screened the myeloma registry of our institution for eMM relapses and identified 24 cases among 357 patients (pts). Only 8% of eMM relapses occurred after initial therapy, but 54% occurred after third-line or subsequent therapy. Baseline molecular cytogenetics revealed high-risk features in 10 of 19 evaluable patients. Most frequently, eMM presented as soft tissue (67%) and organ involvement (25%) or malignant effusion (12.5%). Incidence of leptomeningeal/CNS involvement was 21%. At eMM relapse, bone marrow infiltration was absent in 46% and low in 21%. Ten eMM biopsies were available showing increased proliferation, i.e., Ki-67 of 67% (range, 30–90%) of all cancer cells. Pts received radiation therapy, dose-intense chemotherapy, novel agents, and allogeneic SCT resulting in an overall response rate of 54%. Median progression-free survival was 2 (95% CI 0.08–3.92) and median overall survival 7 months (95% CI 3.56–10.43), respectively, with only three patients being alive at 12 months from diagnosis. EMM relapse may present at any anatomical site with frequent CNS involvement. Biological features include increased proliferation and low rate of marrow involvement. Prognosis remains poor despite intensive treatment. Content Type Journal ArticleCategory Original ArticlePages 1-7DOI 10.1007/s00277-012-1414-5Authors Leo Rasche, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyCorinna Bernard, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyMax S. Topp, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyMarkus Kapp, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyJohannes Duell, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyCarmen Wesemeier, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyEugenia Haralambieva, Institute of Pathology, University Wuerzburg, Josef-Schneider Str.6, 97080 Wuerzburg, GermanyUwe Maeder, Tumor Registry, Comprehensive Cancer Center Mainfranken, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyHermann Einsele, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyStefan Knop, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, Germany Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555
Clinical features and prognostic factors of Asian patients with paroxysmal nocturnal hemoglobinuria: results from a single center in China
Fri, 27 Jan 2012 06:53:43 -0000
Abstract Although all patients with paroxysmal nocturnal hemoglobinuria (PNH) have acquired mutations in the phosphatidylinositol glycan class-A(PIG-A)gene, their clinical courses are highly variable. We reviewed 280 PNH cases referred to our hospital from January 1990 through June 2010 to assess clinical presentations, prognostic factors influencing survival, difference among subcategories, and clinical significance of PNH clone size. The overall survival at 10 years after diagnosis estimated by Kaplan–Meier was 77.6%. Both univariate and multivariate analyses identified risk factors affecting survival, including age >40 years, absolute neutrophil count<0.5 × 109 cells/L, development of thrombotic events, evolution to myelodysplastic syndrome or acute myelogenous leukemia, and recurrent infections. The cohort of patients were divided into subcategories of classic PNH, PNH/AA, and PNH-sc/AA based on the recent proposed PNH working clinical classification, hemoglobinuria as the initial symptomatic manifestation was high up to 82.0% in classic PNH subcategory, whereas only as low as 1.4% in PNH-sc/AA subcategory; the frequencies of infectious (26.0%) and bleeding symptoms (14.0%) in classic PNH subcategory were significantly less than those in PNH/AA (25.3% and 51.7%, respectively) and PNH-sc/AA (48.3% and 83.2%, respectively) subcategories. Our results revealed that large PNH clone was associated with increased risks for hemoglobinuria and thrombosis, whereas small PNH clone was associated with bone marrow failure. Thus, this study may shed insights into Chinese PNH patients to set up individually therapeutic regimens. Content Type Journal ArticleCategory Original ArticlePages 1-8DOI 10.1007/s00277-012-1413-6Authors Meili Ge, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of ChinaXingxin Li, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of ChinaJun Shi, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of ChinaYingqi Shao, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of ChinaYizhou Zheng, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of China Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555
Serum ferritin levels and endocrinopathy in medically treated patients with β thalassemia major
Fri, 27 Jan 2012 06:53:43 -0000
Abstract The association between iron overload indices and pathology of the heart and liver in transfusion-dependent patients with β thalassemia major (TM) has been extensively studied. Nonetheless, data on endocrine disease remains limited. This was a cross-sectional study of 382 TM patients treated with regular transfusions and desferrioxamine at the Thalassemia Center in Dubai, UAE. Retrieved data included demographics, splenectomy status, steady-state serum ferritin levels, and the presence of endocrinopathies (diabetes mellitus, hypothyroidism, hypoparathyroidism, and hypogonadism). Multivariate logistic regression analyses were used to determine which variables were independently associated with the occurrence of each endocrinopathy. The mean age of patients was 15.4 ± 7.6 years, with an equal sex distribution. The mean serum ferritin level was 2597.2 ± 1976.8 μg/l. The frequencies of specific endocrinopathies were diabetes mellitus (10.5%), hypothyroidism (6.3%), hypoparathyroidism (10.5%), and hypogonadism (25.9%). On multivariate logistic regression analysis, patients with a serum ferritin level >2,500 μg/l, but not >1,000–2,500 μg/l, were 3.53 times (95% CI 1.09–11.40) more likely to have diabetes mellitus, 3.25 times (95% CI 1.07–10.90) more likely to have hypothyroidism, 3.27 times (95% CI 1.27–8.39) more likely to have hypoparathyroidism, and 2.75 times (95% CI 1.38–5.49) more likely to have hypogonadism compared to patients with a serum ferritin level ≤1,000 μg/l. However, splenectomized patients with serum ferritin levels ≤2,500 μg/l had comparably high rates of all endocrinopathies as patients with serum ferritin levels >2,500 μg/l. Endocrinopathy is common in TM patients treated with desferrioxamine therapy, especially in patients with serum ferritin levels >2,500 μg/l or those splenectomized. Content Type Journal ArticleCategory Original ArticlePages 1-8DOI 10.1007/s00277-012-1412-7Authors Khawla M. Belhoul, Thalassemia Center, Al Wasl Hospital, Dubai, United Arab EmiratesMaisam L. Bakir, Thalassemia Center, Al Wasl Hospital, Dubai, United Arab EmiratesMohamed-SalahEldin Saned, Thalassemia Center, Al Wasl Hospital, Dubai, United Arab EmiratesAhmed M. A. Kadhim, Thalasse
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Features of extramedullary myeloma relapse: high proliferation, minimal marrow involvement, adverse cytogenetics: a retrospective single-center study of 24 cases
Mon, 30 Jan 2012 06:32:07 -0000
Abstract Extramedullary (e) relapse in multiple myeloma (MM) has an adverse prognosis, but knowledge concerning biological features and preferred treatment is scarce. We screened the myeloma registry of our institution for eMM relapses and identified 24 cases among 357 patients (pts). Only 8% of eMM relapses occurred after initial therapy, but 54% occurred after third-line or subsequent therapy. Baseline molecular cytogenetics revealed high-risk features in 10 of 19 evaluable patients. Most frequently, eMM presented as soft tissue (67%) and organ involvement (25%) or malignant effusion (12.5%). Incidence of leptomeningeal/CNS involvement was 21%. At eMM relapse, bone marrow infiltration was absent in 46% and low in 21%. Ten eMM biopsies were available showing increased proliferation, i.e., Ki-67 of 67% (range, 30–90%) of all cancer cells. Pts received radiation therapy, dose-intense chemotherapy, novel agents, and allogeneic SCT resulting in an overall response rate of 54%. Median progression-free survival was 2 (95% CI 0.08–3.92) and median overall survival 7 months (95% CI 3.56–10.43), respectively, with only three patients being alive at 12 months from diagnosis. EMM relapse may present at any anatomical site with frequent CNS involvement. Biological features include increased proliferation and low rate of marrow involvement. Prognosis remains poor despite intensive treatment. Content Type Journal ArticleCategory Original ArticlePages 1-7DOI 10.1007/s00277-012-1414-5Authors Leo Rasche, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyCorinna Bernard, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyMax S. Topp, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyMarkus Kapp, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyJohannes Duell, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyCarmen Wesemeier, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyEugenia Haralambieva, Institute of Pathology, University Wuerzburg, Josef-Schneider Str.6, 97080 Wuerzburg, GermanyUwe Maeder, Tumor Registry, Comprehensive Cancer Center Mainfranken, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyHermann Einsele, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, GermanyStefan Knop, Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, Germany Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555
Clinical features and prognostic factors of Asian patients with paroxysmal nocturnal hemoglobinuria: results from a single center in China
Fri, 27 Jan 2012 06:53:43 -0000
Abstract Although all patients with paroxysmal nocturnal hemoglobinuria (PNH) have acquired mutations in the phosphatidylinositol glycan class-A(PIG-A)gene, their clinical courses are highly variable. We reviewed 280 PNH cases referred to our hospital from January 1990 through June 2010 to assess clinical presentations, prognostic factors influencing survival, difference among subcategories, and clinical significance of PNH clone size. The overall survival at 10 years after diagnosis estimated by Kaplan–Meier was 77.6%. Both univariate and multivariate analyses identified risk factors affecting survival, including age >40 years, absolute neutrophil count<0.5 × 109 cells/L, development of thrombotic events, evolution to myelodysplastic syndrome or acute myelogenous leukemia, and recurrent infections. The cohort of patients were divided into subcategories of classic PNH, PNH/AA, and PNH-sc/AA based on the recent proposed PNH working clinical classification, hemoglobinuria as the initial symptomatic manifestation was high up to 82.0% in classic PNH subcategory, whereas only as low as 1.4% in PNH-sc/AA subcategory; the frequencies of infectious (26.0%) and bleeding symptoms (14.0%) in classic PNH subcategory were significantly less than those in PNH/AA (25.3% and 51.7%, respectively) and PNH-sc/AA (48.3% and 83.2%, respectively) subcategories. Our results revealed that large PNH clone was associated with increased risks for hemoglobinuria and thrombosis, whereas small PNH clone was associated with bone marrow failure. Thus, this study may shed insights into Chinese PNH patients to set up individually therapeutic regimens. Content Type Journal ArticleCategory Original ArticlePages 1-8DOI 10.1007/s00277-012-1413-6Authors Meili Ge, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of ChinaXingxin Li, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of ChinaJun Shi, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of ChinaYingqi Shao, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of ChinaYizhou Zheng, State Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020 People’s Republic of China Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555
Serum ferritin levels and endocrinopathy in medically treated patients with β thalassemia major
Fri, 27 Jan 2012 06:53:43 -0000
Abstract The association between iron overload indices and pathology of the heart and liver in transfusion-dependent patients with β thalassemia major (TM) has been extensively studied. Nonetheless, data on endocrine disease remains limited. This was a cross-sectional study of 382 TM patients treated with regular transfusions and desferrioxamine at the Thalassemia Center in Dubai, UAE. Retrieved data included demographics, splenectomy status, steady-state serum ferritin levels, and the presence of endocrinopathies (diabetes mellitus, hypothyroidism, hypoparathyroidism, and hypogonadism). Multivariate logistic regression analyses were used to determine which variables were independently associated with the occurrence of each endocrinopathy. The mean age of patients was 15.4 ± 7.6 years, with an equal sex distribution. The mean serum ferritin level was 2597.2 ± 1976.8 μg/l. The frequencies of specific endocrinopathies were diabetes mellitus (10.5%), hypothyroidism (6.3%), hypoparathyroidism (10.5%), and hypogonadism (25.9%). On multivariate logistic regression analysis, patients with a serum ferritin level >2,500 μg/l, but not >1,000–2,500 μg/l, were 3.53 times (95% CI 1.09–11.40) more likely to have diabetes mellitus, 3.25 times (95% CI 1.07–10.90) more likely to have hypothyroidism, 3.27 times (95% CI 1.27–8.39) more likely to have hypoparathyroidism, and 2.75 times (95% CI 1.38–5.49) more likely to have hypogonadism compared to patients with a serum ferritin level ≤1,000 μg/l. However, splenectomized patients with serum ferritin levels ≤2,500 μg/l had comparably high rates of all endocrinopathies as patients with serum ferritin levels >2,500 μg/l. Endocrinopathy is common in TM patients treated with desferrioxamine therapy, especially in patients with serum ferritin levels >2,500 μg/l or those splenectomized. Content Type Journal ArticleCategory Original ArticlePages 1-8DOI 10.1007/s00277-012-1412-7Authors Khawla M. Belhoul, Thalassemia Center, Al Wasl Hospital, Dubai, United Arab EmiratesMaisam L. Bakir, Thalassemia Center, Al Wasl Hospital, Dubai, United Arab EmiratesMohamed-SalahEldin Saned, Thalassemia Center, Al Wasl Hospital, Dubai, United Arab EmiratesAhmed M. A. Kadhim, Thalasse
Sites:
BloodLine: BloodLine is dedicated to furthering and enhancing the fields of hematology and oncology through the presentation of knowledge in the following manners: the publication of original clinical and laboratory research; the creation of comprehensive reference works that are continuously useful to...Atlas of hematology: The Atlas of Haematology contains about 700 illustrations of blood and bone marrow cells
Atlas of Hematology: CDROM Atlas of hematology with 1500 color images, by Prof. John Meletis. Range of examples available online.
Atlas of Hematology: Contains images of blood cells with descriptions.
Aurea R. Tomeski, M.D.: Dr. Tomeski - Internal Medicine - Hematology - Oncology
BloodMed: BloodMed.com - The global source for hematology education, practice and research
European Federation for Immunogenetics: Website for the European Federation of Immunogenetics.
Factor Replacement by Continuous Infusion: Guide for health professionals who provide continuous infusion clotting factor replacement therapy to individuals with bleeding disorders. Describes theory behind how to prescribe, administer, and monitor replacement therapy.
Family Practice Notebook: Hematology and Oncology: Find chapters about Anemia, Cancer, Coagulopathy, Examination, Hemoglobin, Hemolysis, Histiocytosis, Leukemia, Lymph, Marrow, Platelet, Procedure, Sarcoma, Symptom Evaluation and Vascular. Related chapters from other specialties include Cardiovascular, Dermatology, Endocrinology, Otolaryngology,...
General Practice Notebook - Haematology: Coverage of this medical speciality.
Haem.net: Haem.net - The Web Journal of Laboratory Haematology to serve the educational needs of anyone with an interest in Laboratory Haematology in the U.K. and overseas
Haematological Malignancy Diagnostic Service: The diagnosis of leukaemia, lymphoma, myeloma and related blood disorders by cellular and molecular investigation. Contents include the current classification of the myeloproliferative and lymphoproliferative disorders, morphology and cytochemistry, and diagnostic procedures including antibody ba...
Haematology: Haematology & Pathology Education Website for medical laboratory officer`s student or a clinical haematologist in training. Contains an Interactice questions and answers style haematology atlas set of slides. its an online hematology book. particularly useful for hematologists and pathologist...
Hematology: Wikipedia article describing branch of medicine, related diseases, tests, and treatments.
Hematology and Oncology Associates Of Virginia: Virginia Cancer Institute is dedicated to the highest quality medical care for the treatment of diseases of the blood (hematology) to the long-term management of cancer for our patients .
Hematology jobs: Hematology jobs at Physician Employment with automatic email updates.
Hematology, by Ellen C. Ebert, MD: Grant-funded research report which requires the Adobe Acrobat Reader to view.
Hematopathology Correlative Pathology Course: From the UAB Department of Pathology, Birmingham, Alabama.
Machaon Diagnostics: Clinical reference laboratory developing products for diagnosis and monitoring of hemostatic and thrombotic conditions.
Ortho-WIRE: Educational resource for transfusion medicine applications in immuno-hematology, blood group serology, and hemolytic disease of the newborn.
The Vanderbilt Hemostasis-Thrombosis Clinic: The Vanderbilt Hemostasis-Thrombosis Clinic provides comprehensive care for inherited disorders of bleeding or coagulation. Committed to patient care, education, and clinical research, we have over 75 years of experience caring for people with hemophilia, thrombophilia, and other blood disorders.
